Multicenter Experience With Catheter Ablation for Ventricular Tachycardia in Lamin A/C Cardiomyopathy
Saurabh Kumar1, Alexander F A Androulakis1, Jean-Marc Sellal1
1From the Cardiovascular Division, Brigham and Women's Hospital, Boston, MA (S.K., S.H.B., B.A.K., R.M.J., G.F.M., N.K.L., W.G.S., U.B.T.); Department of Cardiology, Leiden University Medical Centre, The Netherlands (A.F.A.A., K.Z.); Hôpital Cardiologique du Haut-Lévêque (CHU), Bordeaux-Pessac & L'Institut de RYthmologie et Modélisation Cardiaque (LIRYC), Institut Hospitalo-Universitaire (IHU), Bordeaux, France (J.-M.S., F.S.); Centre Hospitalier Universitaire de Nancy (J.-M.S.); Toulouse University Hospital, Rangueil, Toulouse (P.M., A.R.); Département de Cardiologie, Hôpital Pitié-Salpêtrière, AP-HP (J.-M.S., F.S., E.G., X.W.); Cardio-myogenetic, Department of Biochimie and INSERM U582, University Hospital Pitié-Salpêtrière, AP-HP (P.R.); Centre de Référence Maladies Cardiaques Héréditaires, ICAN, Hôpital Pitié-Salpêtrière, Paris (P.C.); and Université de Versailles-Saint Quentin, Hôpital Ambroise Paré, AP-HP, Boulogne-Billancourt, France (P.C.).
Insights
Catheter ablation for ventricular tachycardia (VT) in Lamin A/C (LMNA) cardiomyopathy shows poor outcomes. Recurrence, heart failure progression, and mortality remain high due to challenging basal septal scar and intramural VT origins.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Lamin A/C (LMNA) cardiomyopathy is a genetic heart condition predisposing patients to ventricular arrhythmias.
- Percutaneous catheter ablation is a potential treatment for sustained monomorphic ventricular tachycardia (VT) in this population.
Purpose of the Study:
- To describe the multicenter experience and outcomes of catheter ablation for VT in patients with LMNA cardiomyopathy.
Main Methods:
- Twenty-five LMNA mutation patients with VT underwent catheter ablation across four centers.
- Procedures included mapping, radiofrequency ablation, transcoronary alcohol septal ablation, and surgical cryoablation.
- Outcomes assessed included acute success, VT recurrence, heart failure progression, and mortality.
Main Results:
- Acute success (noninducibility of any VT) was achieved in only 25% of patients after a median of two procedures.
- VT recurrence was observed in 91% of patients within a median follow-up of 7 months.
- Forty-four percent of patients progressed to end-stage heart failure requiring mechanical support or transplant, and 26% died.
Conclusions:
- Catheter ablation for VT in LMNA cardiomyopathy is associated with high rates of recurrence, heart failure progression, and mortality.
- The intramural nature of basal septal scar and VT origin presents significant challenges for successful ablation in this patient group.
Background:
Lamin A/C (LMNA) cardiomyopathy is a genetic disease with a proclivity for ventricular arrhythmias. We describe the multicenter experience with percutaneous catheter ablation of sustained monomorphic ventricular tachycardia (VT) in LMNA cardiomyopathy.
Methods And Results:
Twenty-five consecutive LMNA mutation patients from 4 centers were included (mean age, 55±9 years; ejection fraction, 34±12%; VT storm in 36%). Complete atrioventricular block was present in 11 patients; 3 patients were on mechanical circulatory support for severe heart failure. A median of 3 VTs were inducible per patient; in 82%, mapping was consistent with origin from scar in the basal left ventricle, particularly the septum, but also basal inferior wall and subaortic mitral continuity. After multiple procedures (median 2/patient; transcoronary alcohol in 6 and surgical cryoablation in 2 patients), acute success (noninducibility of any VT) was achieved in only 25% of patients. Partial success (inducibility of a nonclinical VT only: 50%) and failure (persistent inducibility of clinical VT: 12.5%) was attributed to intramural septal substrate in 13 of 18 patients (72%). Complications occurred in 25% of patients. After a median follow-up of 7 months after the last procedure, 91% experienced ≥1 VT recurrence, 44% received or were awaiting mechanical circulatory support or transplant for end-stage heart failure, and 26% died.
Conclusions:
Catheter ablation of VT associated with LMNA cardiomyopathy is associated with poor outcomes including high rate of arrhythmia recurrence, progression to end-stage heart failure, and high mortality. Basal septal scar and intramural VT origin makes VT ablation challenging in this population.
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